AIM: Paragangliomas are neural crest-derived neuroendocrine tumors, originating from paraganglia, which are dispersed neuroendocrine organs characterized by catecholamine and peptide-producing cells. With an annual incidence estimated at 1/100,000, paragangliomas represent 10% of catecholamine secreting tumors. MATERIAL OF STUDY: We report a case of a 76-year-old man who was submitted to a subtotal gastrectomy with omentectomy and gastrojejunal anastomosis. The Hystologic exam has revealed an ulcerative polypoid gastric carcinoma with cell poorly cohesive and infiltration of the muscular gastric wall and an incidental parietal gastric lesion which was a paraganglioma with immunocytochemical investigations positive for NSE and negative for CD117, S100, CD34 e SMA. DISCUSSION: Pheochromocytoma indicates exclusively tumors arising from the adrenal medulla, while the extra-adrenal paraganglioma suggests tumors of the chromaffin cells with other locations. Gastric or paragastric localization, as in our case, is very rare for these neoplasms, and in literature there are only isolated case reports. Genetical predisposition is observed in 30% of these tumors and can be responsible of hereditary disease characterized for differences in tumor distribution, catecholamine production, risk of metastasis, and association with others types of tumors. CONCLUSION: In asymptomatic patients and when biochemical and clinical suspicion of neuroendocrine tumor is strong, you have to perform anatomical and functional investigations to detect these neoplasms. The first line treatment for resectable tumors is complete surgical resection, that can be performed with open surgery or laparoscopic technique. Surgical therapy is also indicated to palliative intent when a complete eradication of disease is not achievable for metastatic status of malignancies.